I write as an outlet, as two of our children battle cancer, positive for Li-Fraumeni Syndrome. I cannot say how often I have heard the phrase, "I cannot even imagine..." but hope to give a glimpse of what this is like...and to reassure you that it is not all bad, despite the challenges.

Thursday, April 25, 2013

In the Begining...

So, Dan has been saying for a couple of weeks now that I
need to give sort of a background of our story…a thumbnail of how we got here,
because some on this blog are not on Caringbridge.In truth, I have been dodging this task (but
in fairness, it is not like I have been eating bonbons and watching reality TV
or anything so frivolous)

So, how did we get here, in 10,000 words or
less?Uggh….Bonbons and reality TV is
sounding pretty good about now.

Our cancer story began in late 2003, once upon a time, when
Dan and I were younger, much less medically experienced and simply enjoying our
family.We were busy, I thought, with
three small children, but a ‘good busy’ and we had a blessed life.

That whole frame of reference shifted, just changing
Lauren’s diaper a bit before Christmas.I noticed that she had begun to get some hair…down there.I had a 15 month
well visit on the calendar for her and so I planned to mention it to our
pediatrician when we went in.We didn’t
see her regular doctor that day at the practice (it was nearing on Christmas), but the substitute pediatrician
blamed it on hormones passed to Lauren from
my birth control pills, because I was still nursing.Lauren was my last baby, and Dan still
brought her in to me first thing each morning.Feeling guilty and responsible for harming her, I never nursed her
again.

I did, however, call my OB, to let him know (after all, he had prescribed
the pills knowing that I hadn’t completely weaned her yet).He asked me to bring Lauren in to see him
that same day.After examining her, he
encouraged me to see a pediatric endocrinologist by the first of the year and
to get some answers (What?Getting into
a specialist might take months, even without the holidays) He was emphatic that
my nursing her had nothing to do with
this, and strongly suspected some endocrine issue, maybe with the pituitary
gland. He warned that Lauren might continue to develop, even start menstruating
(What??!!! She is in diapers!!) He told
me not to worry, but to get some answers.He also offered to help me get the consult, if I had any problems
getting in.First of the year…

This whisper of urgency was a message from God.

The specialist we saw initially examined her, but just wanted
us to come back in 6 months.He wasn’t
even going to draw any labs.My OB’s
words stayed with me, and I pushed.Finally, the endocrinologist agreed to draw blood, but assured us that
he had seen this before, not to worry, and that we would see him in the
summer.

He called a few days later, with results that he did not
expect, but that he failed to explain, despite our many questions.He ordered another test, again, with assurances
that knew what it would likely be…although he never explained what that precisely
was. We repeated this pattern several times, for several different tests.

Finally, by early March, I took Lauren in for the latest
test, an abdominal ultrasound.Dan and I
had decided that after this test, we wanted a new doctor, because this man,
while very smart, was not a good fit for us.We needed to understand what he was looking for, and what he was ruling
out.

We decided that Dan would take half a day off of work, to be
with the boys and I would run in to Rainbow for the ultrasound, which was
non-invasive and easy.In the darkened room, I chatted with the tech,
asking questions about what we were looking at, because when I was pregnant, I
could never tell the difference between a foot and a face, much less determine who
the child looked like.

“… so, that is her
adrenal gland?Interesting…”On to the other side.

“Is that her other adrenal gland?”

No.You apparently
are not supposed to be able to see it. This information was starting to take
root in my mind.But the tech then said
that we needed a full bladder to look at her uterus or her ovaries, I forget
which.Could I maybe get her to drink
some juice and fill her bladder over the next half an hour and she would be
back?

Alone in the room, we looked at the colorful mobile that hung
above the examining table, read a book to Lauren, plying her with juice (I
doubted that this would really work…in my experience, a full, uncomfortable
bladder for a baby means an empty bladder and a full diaper).I considered the two adrenals, the discrepancy
in size and thought about how Dan would laugh at me for the fleeting thought
that it was a tumor.How ridiculous,
because she was a baby.We would
certainly be laughing at this mama by dinnertime for being so silly.

But half an hour later, when the tech came back, she was not
alone.There was a full posse of medical
backup…oncologist, social worker, radiologist…I forget who all else, but from
my current perspective, I suspect the rest were underlings in tow (until we
were well into this journey, I did not understand that there were levels of
doctors, and doctors in training…all white coats are not created alike.)

There was a mass of people in the room.

There was also a mass, in
my daughter.

They were admitting
her, because one type of adrenal tumor causes dangerously high blood pressure
and she could stroke out.Did I need to
make some calls?The social worker would
mind Lauren, and guide me over to admitting.We would then be going to the oncology floor. She was going to be
sedated for a CT scan.I was informed of
these things, told of this plan, not asked about it.I think that this was probably a very good
thing, having it all decided for me, because my brain had entirely stopped
functioning.

This was the moment that I realized that I would never be going back to Kansas.

The social worker went with me and Lauren to a bank of pay phones,
where I called Dan ‘…come meet me in pediatric oncology.Yes, that means cancer.Get someone to stay with the boys…my sister,
or a friend in our neighborhood…but you need to come.Now.’ It became a blurry nightmare…

Horrible call.Horrible day.To this day, I
cannot walk past that bank of payphones without being in that moment, stomach
lurching.And they remain there for me
to walk past, nearly every time I am in the hospital.It never gets better.Those pay phones take me there, every single
time.

As it turned out, Lauren did not have the blood pressure
issue with her tumor.She had another,
rarer, kind of mass (3-4 cases in 10 million as I understand it).We were discharged after a sleepless night on
RB2, one that I have next to no memory of, except that I left with a pounding
headache, likely from not eating, not sleeping, and crying an awful lot.

While we waited a week for surgery to remove the tumor and
entire gland, I thought about how fortunate we were that this happened at her
age.Because, had she been a bit older,
she would have been potty trained, and I would not have noticed her symptoms so
easily.Her ravenous appetite, I chalked
up to an impending growth spurt.Many
children get vaguely moody.The one
thing that really got my attention was the pubic hair.And had I not seen it, especially when my OB
would have reason to weigh in on it, well, we might have been in a whole
‘nuther boat.

A week later, when we came in for surgery, I remember
changing her diaper and looking at her belly.She looked so pink, so happy, so perfect.I could not imagine handing her over to the surgeons, for them to cut her
open, because she appeared so healthy.It
was hard to remember that she was sick, and it was very serious because she was a happy child, in no pain.I really wished that a bikini wax would have
fixed it.

The surgery took several hours.It felt longer, because as I now understand
these things, it always does.But Lauren
did well.Within days, we ended up
chasing her around the unit with an IV pole on Rainbow 2, wheeling her around
in a wagon.She played in the playroom
incessantly, partial to a kitchen set that they had there.We were especially encouraged by her bounce,
probably because we were absolutely spent with worry.

Clean margins, completely resected.When the pathology came in mixed, they explained
that there were several factors that were encouraging…but the most concerning,
was that it was pleomorphic, meaning under the microscope, there many different
looking cells, which suggested an aggressive tumor.

For this rare type of cancer, Adrenal Cortical Cancer (ACC),
there was no effective chemotherapy available.We could only wait and watch.They would scan her, and do blood tests, measuring the testosterone
level.If the cancer came back, wherever
it raised its ugly head, it would produce testosterone, which was what caused
the pubic hair to grow, and the appetite.This was something that we could detect at very low levels in her blood,
likely before it produced any symptoms.This
is how we would know.And then we would
go looking for it.

When we came home, Dan turned to me, and said that he had to
leave for a bit.I was surprised, given
that we had just returned home from a weeklong stint through the bowels of an
emotional hell, the likes of which I could not have imagined.I was personally ready for a nap.

When he returned, his mission was clear.He had been to Toys R Us and picked up a
kitchen set for Lauren.He noticed how
much she enjoyed the one in the hospital, and really wanted her to have that same
enjoyment at home.It was a celebration
of her recovery, and reward to her for being such a trooper.This is just how my husband is.I love him for it, and weep at the thought of
how much he cares and looks out for our children.

When we came in to RBC for our first outpatient visit after
surgery, the oncologist had a long talk with us.She let us know that given Lauren’s rare
tumor and young age, we should consider genetic testing.The overwhelming odds, given the ACC, favored
her having a genetic predisposition to cancer which was even more rare than the
very rare tumor.It was called
Li-Fraumeni Syndrome.It is a failure of
the tumor suppressor gene p53, so she would more likely develop other cancers,
different cancers.All cancers.

And if she had it, we
should consider testing the boys.And
ourselves. There was a 50% chance that
we were also carriers. Would we mind
sending her tumor to St. Jude’s for testing?‘What were we going to do with it?Put it in a jar on the mantle?Of
course, send it, by all means.’

This,
incidentally, might have been the very beginnings of my stress driven snark.

So, that day, we went from being cautiously pleased with
Lauren’s surgical recovery and the mostly positive news out of pathology, feeling
that we could beat this, to learning that we were likely only beginning our
troubles, which could be vast.

The genetic testing takes time, and for a month, I could
only look at my children and instead of entertaining typical maternal thoughts,
imagining the people that they would become (What college would they go to?
What will their spouse be like?Would
they have children of their own?What
career might they take up?) I worried about a life in hospitals and the cancers
that the three of them might develop.It
was a very long time in limbo.

Internet research, for the record, is not a good pastime at
such a point in your journey.LFS is no
picnic, I rapidly discovered.It runs in
families, and is devastating.There is
no cure, and only recently was there a way to test to see which family members
would be affected by carrying a mutation in their p53 gene.I cannot imagine how, in the absence of
science, families processed this, making sense of the frequent and returning
cancers in their family, of every variety.They must have felt cursed by God.They might still.

When we returned to RBC a month later, the genetic results
still were not back.The oncologist,
knowing our anxiety, kindly called down to St Jude’s and got results faxed
over.She excitedly told us that we were
somehow on the skinny side of the odds…Lauren did not have the
mutation.This was a fluke…Bad luck…A lightening strike…Pretend that this never happened.Carry on.Congratulations!

Jubilation and disbelief doesn’t begin to describe how we
felt. In retrospect, we probably should
have stuck with disbelief, but who doesn’t grab on to good news when it is
offered?No one gets a second opinion on
good news.

And as we progressed, drawing labs every month, with
reassuringly low testosterone numbers and scanning her every three months with
clean CT scans, the likelihood of the adrenal cancer returning decreased.My anxiety on scan day never did, but
statistically speaking, if the cancer were to return and spread, it would have likely
done so within the first year.We
scanned her for 18 months and followed her labs, mostly for my own peace of
mind, for several years at increasing intervals.We were moving on.

But despite this generally positive trend, I frequently
wrestled during this time with odd things that happened with Lauren.Changes in her appetite had a sinister feel
to them, a potential symptom of the cancer’s return. (And what child doesn’t
have these sorts of changes as they grow?) I tried to tamp down my worries and reminded
myself that we had it beat.And it was
only a lightning strike...A fluke...Bad luck...

I am ashamed to say that more than once, I panicked as I
changed her diaper…spotting a black hair or two on her nether parts.The sick feeling, as my stomach dropped and
my mind raced to very ugly places, fearing that the cancer was back…well, it
fortunately disappeared upon closer inspection.

I cursed our black dog Max, and my apparent lax housekeeping
skills.It was only stray dog hair down
there, hair that easily wiped away.Yes,
this happened more than once, but my reaction was always the same.I can laugh about it now, but in that moment,
you know deep in your soul, that it can all change just that quickly. And while the hair is easily wiped away, the
fear is not.

So, I vowed to vacuum more diligently, to enjoy my
blessings, and to try, with much effort, to relax.I do recognize the paradox in that statement,
but it was an effort…one that was required in order to make a new habit. To relax.I was determined to not allow this episode to
change our happiness.

Back in the day, we didn’t have cell phones and
voicemail.We waited for important
calls, chained to our home, paralyzed with the wait.When there were lab results, or scan
results, this didn’t help me in my vow to enjoy my blessings, or offer any
assistance with my effortto relax about
the results.

And while my time on
the phone with doctors and nurses naturally had to be a priority, Lauren was
oblivious of this because of her young age.The boys, and Alex in particular, were not oblivious.

Alex had questions, as all children do.Especially on occasions when I was talking on
the phone to various medical folk, and I would scold him and Brent impatiently,
for squabbling over a toy, interrupting and distracting me from a rather
significant conversation.(They were 4
and 6, so, it is not surprising that they would occasionally need outside
intervention, a peacekeeper, or negotiator)But, I felt that I had to explain to them what was happening in our
family and why they had to be especially good while I was talking on the phone
to doctors.

Many people would try to protect their children from the
hard reality of this all.If I could, I
probably would have tried.But, for me,
it seemed that Alex’s little world would make most sense if he understood what
was going on. He would understand me,
and trust me.It was a philosophical
position, one of honesty, despite his young age.I have never backed down from that decision.And it has not always been easy.

After I explained that Lauren was sick, and that I needed to
talk to her doctors, who were trying very hard to fix her up, Alex asked me if
she was going to be all right.He asked
me, point blank, if she would die.

It was quite the loaded question out of a six year old.

I was honest.I told
him that I didn’t know.And it was
perhaps the hardest sentence that I have ever been required to spit out. It broke my heart saying it.Almost in tears, I nearly choked on the
words, and the awful reality of what those words represented.Speaking the truth to him, I could no longer
deny the possibility in my own mind.

Not to spoil the ending or anything, but I have had to say
these words more than once to Alex.And
it does not get any easier; you only think less about cushioning the truth in
some fluff.Because, God forbid, if it
didn’t work out, which I could not control, I could not have thatabsolute travesty compounded by Alex’s
mistrust of me and my words.I knew that
if I lost Lauren, I would also lose Alex, unless I was very clear and very
honest in that particular moment.

This was instantly distilled in my mind on that day, sitting on
the steps of our little home, looking into his questioning eyes.

About Me

Our pediatric cancer adventure was complicated by the discovery of a genetic mutation (Li-Fraumeni Syndrome) which predisposes two of my children to all forms of cancer. While Brent, now 16, battled osteosarcoma (bone cancer), we found that Lauren, now 13, had a rare brain tumor. Since our initial problems in 2011, Brent developed two subsequent cancers (Metastatic melanoma and AML--a leukemia that required bone marrow transplant) and Lauren had a second brain tumor removed.

I share both the challenges and the blessings of this life, pretty freely.